Behcet's Disease

Basics of Behcet's Disease

Behcet's disease is a condition that affects the inner lining of the mouth and genitals, and the small blood vessels throughout the body. Prognosis There is no cure for Behcet's disease, but the symptoms usually can be controlled. Behcet's disease can affect many parts of the body. It can be mild or serious, depending on which areas are affected.

Incidence

Behcet's disease is a rare disease that affects about 15,000 to 20,000 people in the United States. It usually affects young adults in their 20s or 30s. It affects more women than men.

Acquisition

The cause of Behcet's disease is unknown. Researchers think either a genetic cause (inherited from parents) or a virus is responsible for Behcet's disease.

Causes

The cause of Behcet's disease is unknown. Researchers think either a genetic cause (inherited from parents) or a virus is responsible for Behcet's disease.

Credits

Some of this material may also be available in an Arthritis Foundation brochure. Adapted from the pamphlet originally prepared for the Arthritis Foundation by H. Ralph Schumacher, M.D. Peter Hasselbacher, M.D. This material is protected by copyright.

Symptoms

Common symptoms

  • Mouth ulcers: These sores can occur on your tongue, and on the inside of your lips and cheeks. They can be painful and may make it hard to eat or swallow. They usually last for one to two weeks, but can occur again and again.
  • Eye problems: These can be the most serious symptoms of Behcet's disease. The disease can cause pain, redness and swelling of eye tissues. This is called uveitis. The disease can also affect the retina (the inner lining of the eye). If not treated, this can cause blindness.
  • Genital ulcers: These appear less often than the mouth sores. They are sometimes mistaken for herpes, but are not caused by the herpes virus.
  • Skin bumps: Reddish bumps and sores that resemble acne can occur on your trunk, arms, and legs.

Less common symptoms

  • Arthritis: Joint pain and swelling, especially in your knees, ankles, elbows, and wrists. It usually does not cause joint deformity.
  • Abdominal pain and tenderness, and blood in your stool. This is sometimes mistaken for Crohn's disease, a condition that affects the bowel.
  • Fever, a stiff neck, and headaches: This happens if the condition affects your brain and nervous system.
  • Phlebitis: This is swelling of veins, usually the veins in your legs. The area around the vein may become painful and tender. The skin around the area may become red and may feel warm. It may cause blood clots to form, which can cause serious problems.
  • Behcet's disease can also affect the lungs or heart. In rare cases, this can be life-threatening.

Diagnosis

Behcet's disease may be hard to diagnose, because it often takes a while for all the symptoms to appear. When symptoms occur, a diagnosis can usually be made based on a physical examination and your medical history. A skin test, called the Behcetin Reaction can also confirm if you have the condition. For this test, the doctor will prick your skin with a small needle. If a nodule or sore develops at the site of the prick one to two days later, then the doctor will be able to determine if you have Behcet's. Your doctor may also order tests to look for eye problems or problems with the lining around the brain. Some blood tests may also be helpful. If you have the classic symptoms of recurring mouth sores, along with uveitis, recurring genital sores, skin sores and/or a positive Behcetin Reaction, then you most likely have the condition.

Treatment

There is not yet a cure for Behcet's disease, but its symptoms can be treated and managed. When treatment is effective, many people with Behcet's enter a quiet stage of the disorder after an active phase of a few years. With new medicines available, the outlook is good for maintaining vision and avoiding life-threatening problems.

Health Care Team

Because various parts of your body may be affected, you may need to see different types of doctors, such as an eye doctor, skin doctor, and arthritis specialist.

Medications

Depending on your symptoms, your doctor may recommend one or more of the following medicines:

  • Corticosteroid creams applied directly to the mouth, skin, or eyes to reduce pain and swelling. These medicines are related to cortisone, a natural body hormone.
  • Corticosteroid pills, such as prednisone. These help reduce pain and swelling throughout the body. These are powerful medicines. If used for a long period of time, they can cause side effects, such as osteoporosis and bone fractures.
  • Pain-relieving rinses for mouth ulcers.
  • Nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin or ibuprofen to relieve pain and swelling.
  • Colchicine, a medicine used to treat gout, to reduce active pain and swelling.
  • Cytotoxic drugs, such as chlorambucil (Leukeran) or azathioprine (Imuran). These are usually given to people who have eye or brain involvement. These drugs reduce pain and swelling. They also slow down the disease process. They are powerful medicines and can have serious side effects. If you are taking these medicines, you will have regular blood tests to check for side effects.
  • Experimental drugs are sometimes tried if other drugs do not work. For example, the drug cyclosporin, which is given to people who have organ transplants, may be effective in treating people with Behcet's disease.
  • Work with your doctor to decide which medicine is best for you. Ask your doctor about side effects and how to take the medicine. If you notice any side effects, call your doctor.